Griffin J H
Semin Thromb Hemost. 1984 Apr;10(2):162-6. doi: 10.1055/s-2007-1004419.
The major clinical importance of plasma protein C is attested to by the strong association between inherited protein C deficiencies of half normal levels and recurrent venous thromboembolic disease. Homozygous protein C deficient individuals do not survive beyond infancy without continuous therapeutic intervention. The spectrum of protein C deficiency is becoming broader and includes patients with both abnormal molecules and half normal levels of functionally active molecules. Rarely, a few young adults with thrombosis have been identified with protein C levels below 25%. Studies of protein C activity have been hampered until the very recent developments of functional assays of plasma protein C. Application of these assays to a wide variety of clinical situations involving thrombotic complications is just beginning and may lead to an explosive proliferation of new data that should prove most fascinating and give much further insight into the contributions of protein C in the regulation of thrombosis.
遗传性蛋白C水平降至正常一半与复发性静脉血栓栓塞性疾病之间存在密切关联，这证明了血浆蛋白C在临床上的重要性。纯合子蛋白C缺乏的个体若不进行持续治疗干预，在婴儿期之后便无法存活。蛋白C缺乏的范围正在扩大，包括分子异常以及功能活性分子水平降至正常一半的患者。很少有血栓形成的年轻成年人被发现蛋白C水平低于25%。直到最近血浆蛋白C功能测定方法出现，蛋白C活性的研究才受到阻碍。将这些测定方法应用于涉及血栓形成并发症的各种临床情况才刚刚开始，可能会引发新数据的爆发式增长，这些数据将极具吸引力，并能让我们更深入地了解蛋白C在血栓形成调节中的作用。
